Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005
Primary immunodeficiency diseases: An update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005
复制标题
DOI：
10.1016/j.jaci.2005.12.1347
复制
发表时间：
2006-04-01
影响因子：
14.2
通讯作者：
Geha, RS
中科院分区：
文献类型：
--
作者：
Notarangelo, L;Casanova, JL;Geha, RS
虽然相对罕见，但原发性免疫缺陷病（PID）为了解免疫系统的功能提供了一个很好的窗口。在20世纪60年代后期，对这些疾病及其相关感染和遗传学的观察将免疫系统分为体液免疫和细胞介导的免疫。这些疾病在诊断和治疗方面也是一个挑战。从1970年开始，由世界卫生组织召集的一个委员会为当时已知的PID创建了统一的命名法。从那时起，后来在国际免疫学会联合会的主持下，国际专家委员会每2至3年举行一次会议，以更新PID的分类。在过去的15年中，已经阐明了120多个PID的分子基础。这一更新是该委员会2005年6月在匈牙利布达佩斯举行的最近一次会议的结果，这次会议是在进行了为期两天半的科学讨论之后举行的。作为这项工作的结果，新的实体已经被包括在内，一些PID的命名（特别是各种形式的类别转换重组缺陷，以前称为高IgM综合征）已经改变。
Although relatively rare, primary immunodeficiency diseases (PIDs) provide an excellent window into the functioning of the immune system. In the late 1960s, observations on these diseases, with their associated infections and genetics, bisected the immune system into humoral immunity and cell-mediated immunity. These diseases also represent a challenge in their diagnosis and treatment. Beginning in 1970, a unified nomenclature for the then-known PIDs was created by a committee convoked by the World Health Organization. Since then, and later under the aegis of the International Union of Immunological Societies, an international committee of experts has met every 2 to 3 years to update the classification of PIDs. During the past 15 years, the molecular basis of more than 120 PIDs has been elucidated. This update results from the latest meeting of this committee in Budapest, Hungary, in June 2005, which followed 21/2 days of scientific discussions. As a result of this work, new entities have been included, and the nomenclature of some PIDs (specifically of the various forms of class-switch recombination defects, previously known as hyper-IgM syndromes) has been changed.